Bernard-Soulier Syndrome
- Autosomal Recessive
- Deficiency of Glycoprotein Ib (GpIb) Receptor
- Mucosal Membrane Bleeding
- Menorrhagia
- Easy Bruising
- Large Platelets
- Increased Bleeding Time
- No Platelet Agglutination with Ristocetin
Glanzmann Thrombasthenia
- Autosomal Recessive or Acquired as an Autoimmune Disorder
- Deficiency of Glycoprotein IIb/IIIa (Gp IIb/IIIa) Receptor
- Defective Platelet-to-Platelet Aggregation
- Mucous Membrane Bleeding (Gingival Bleeding)
- Easy Bruising
- Increased Bleeding Time
- Normal Platelet Count
- Avoid NSAIDs
Primary Immune Thrombocytopenia
- Preceding Infection or Vaccination
- Antiplatelet Antibodies
- Often Asymptomatic
- Bleeding
- Petechiae
- Thrombocytopenia
- Increased Megakaryocytes
- Corticosteroids
- Intravenous Immunoglobulin (IVIG)
- Rituximab
- Splenectomy
Thrombotic Thrombocytopenic Purpura (TTP)
- Inhibition or deficiency of ADAMTS 13
- vWF Multimers are Not Degraded
- Microthrombi (and Emboli)
- FAT RN Pentad
- Fever
- Anemia
- Thrombocytopenia
- Renal Dysfunction
- Neurologic Abnormalities (Altered Mental status)
- Plasmapheresis
- Corticosteroids
- Splenectomy
Hemolytic Uremic Syndrome (HUS)
- Children
- Preceded by Acute Diarrheal Illness
- Endothelial Damage
- Microangiopathic Hemolytic Anemia (MAHA)
- Thrombocytopenia
- Renal Failure
- Helmet cells
- LDH
- Dialysis
- Supportive